OBJECTIVES/HYPOTHESIS: To report two cases of primary lymphoepithelial carcinoma in the middle ear and to determine the optimal treatment for such lesions. STUDY DESIGN/METHODS: Case reports and a systematic review of the literature. RESULTS: Primary lymphoepithelial carcinoma in the middle ear is an exceptionally rare condition with only two other cases reported in the literature. There appears to be an association with Epstein-Barr virus infection and in those patients originating from the Guangdong province of China, much as is the case for similar lesions found in the nasopharynx. Piecemeal rather than en bloc excision, in combination with adjuvant radiotherapy, appears to adequately control the disease. CONCLUSIONS: Primary lymphoepithelial carcinoma of the middle ear is a rare lesion, which when treated has a good prognosis.